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G42 Gastroenterology
Biliary Tract Toronto Notes 2019 Primary Biliary Cholangitis (formerly cirrhosis)
Definition
• chronicinflammationandfibrousobliterationofintrahepaticbileductules
Etiology/Epidemiology
• likelyautoimmune(associatedwithSjögren’ssyndrome,scleroderma,CRESTsyndrome,RA, thyroiditis)
• affectsmainlymiddle-agedwomen(M:F=1:9)
Signs and Symptoms
• oftenasymptomatic
• initial symptoms: pruritus, fatigue
• chronic:jaundiceandmelanosis(darkeningskin)andothersignsofcholestasis • end-stage:hepatocellularfailure,portalHTN,ascites
• highincidenceofosteoporosis
Investigations
• increasedALP,GGT;bilirubinrisesinlaterstage
• positiveanti-mitochondrialantibodies(AMA;95%specificityandsensitivity) • elevatedIgM
• increasedserumcholesterol(mildincreaseinLDL,largerincreaseinHDL)
■ may have: xanthelasmas, xanthomas
• liver biopsy confirms diagnosis and stages severity
• normalbileductonMRCPrulesoutbileductobstructionwhichcanmimicPBC
• recentlydescribed“overlap”syndromeswithautoimmunecholangitis,autoimmunehepatitis,sclerosing
cholangitis
Treatment
• drugsthattreattheunderlyingdisease:
■ ursodiol (usual first line treatment)
■ obeticholic acid (particularly if inadequate response to urosiol)
• cholestyramine (for pruritus and hypercholesterolemia)
• calciumandvitaminDforlowbonedensity;bisphosphonatesifosteoporosissevere • monitorforthyroiddisease
• livertransplantifdiseasesevere,progressive
Prognosis
• canbefatal,althoughnotallasymptomaticpatientsshowprogression
Table 20. Primary Sclerosing Cholangitis vs. Primary Biliary Cholangitis
MRCP/ERCP
• Absence of narrowing in PBC
• Narrowing of intra and extrahepatic ducts
in PSC
Endoscopic or Surgical Step-Up Approach for Infected Necrotising Pancreatitis: A Multicentre Randomised Trial
Lancet 2018;391:51-58
Purpose: To compare the outcomes of endoscopic versus the standard surgical removal of infected necrotising pancreatitis.
Study: Randomized controlled trial.
Population: 98 patients with pancreatic or extrapancreatic necrosis were randomized to either endoscopic removal or surgical removal of the infected necrosis.
Outcomes: Major complications and death at 6 mo follow-up.
Results: The primary end-points of mortality and major complications was the same in the two groups, but the endoscopic group had shortened hospital stays and less pancreatic fistulas. Conclusions: Three points are worth emphasizing: (1) necrotising pancreatitis is a severe disease: about 15% in each group died; (2) it is best to wait at least 4 wk after the necrosis has developed to allow it to be encapsulated; (3) the management of severe acute pancreatitis should include experts skilled in therapeutic endoscopy.
Predominant Gender Associated Comorbidities Affected Ducts Investigations
Primary Sclerosing Cholangitis
Male
IBD, especially UC
Both intra- and extra-hepatic
ERCP/MRCP (narrowing and dilatations of ducts visualized)
Primary Biliary Cholangitis
Female
Other autoimmune disorders (Sjögren’s, CREST, RA)
Intrahepatic only
Anti-mitochondrial antibodies, IgM, increased lipids, liver biopsy (absence of duct narrowing on ERCP)
Secondary Biliary Cirrhosis
Definition
• cirrhosisfromprolongedpartialortotalobstructionofmajorbileducts
Etiology
• acquired:post-operativestrictures,chronicpancreatitis,sclerosingcholangitis,stoneinbileduct • congenital:CF,congenitalbiliaryatresia,choledochalcysts
Investigations
• cholangiographyandliverbiopsy
Treatment
• treatobstruction,giveantibioticsforcholangitisprophylaxis
Biliary Colic, Cholecystitis
• seeGeneralSurgery,GS47